Is “unilateral” moyamoya disease different from moyamoya disease?

Abstract

Whether a diagnosis of moyamoya disease is justified in patients with typical angiographic evidence of moyamoya disease unilaterally and normal angiographic findings contralaterally remains controversial. In this study the authors analyzed longitudinal angiographic change, familial occurrence, and basic fibroblast growth factor (bFGF) concentration in the cerebrospinal fluid (CSF) of patients with "unilateral" moyamoya disease. Over a 10-year period, 10 cases of unilateral moyamoya disease were followed using conventional angiography or magnetic resonance angiography. Basic FGF in CSF, obtained from the subarachnoid space of the cerebral cortex during revascularization surgery, was measured in five cases. Among the 10 cases of unilateral moyamoya disease, only one pediatric case showed obvious signs of progression to typical bilateral disease. The other nine cases (including six adults and three children) remained stable throughout follow-up radiological examinations (magnetic resonance angiography) with a mean observation period of 3.5 years. There was no familial occurrence in these cases of unilateral moyamoya disease. Levels of bFGF, which are high in typical moyamoya disease, were low in these patients. The progression from unilateral moyamoya disease to the typical bilateral form of the disease appears to be infrequent. The low levels of bFGF in the CSF of these patients and the lack of familial occurrence strongly suggest that most cases of unilateral moyamoya disease, especially those found in adults, are distinct from typical bilateral moyamoya disease.

Increased prevalence of autoimmune disease in patients with unilateral compared with bilateral moyamoya disease

“…13,16,20 Several studies have suggested that an association exists between MMD and autoimmune diseases, including Type 1 diabetes mellitus, AIG, SLE, APAS, APS, PBC, GD, and HT. 2,7,15,17,18,20,22,23,25 Furthermore, several reports have demonstrated that thyroid autoantibodies may be associated with the moyamoya development. 13,15,16 Our study also indicated that autoimmune abnormalities may be associated with pathophysiological mechanisms of moyamoya vasculopathy formation.…”

“…7,11,14,18 MMD has been reportedly associated with various entities. 2,8,9,13,16,24 Analysis of the coexisting diseases that occur with moyamoya may be helpful in exploring moyamoya pathogeneses.…”

Unilateral Moyamoya Phenomenon with a String-of-beads Appearance in an Elderly Patient with the c.14576G>A Heterozygous Variant of RNF213

“…The possibility of progression in adolescence and beyond appears to be infrequent (4,5). The level of basic fibroblast growth factor in the cerebrospinal fluid is reported to be related to progression (6). It thus is reasonable to consider that the presence of unilateral moyamoya vessels in elderly individuals is attributable to atherosclerosis and that this phenomenon is distinct from moyamoya disease.…”

Moyamoya disease associated with recurrent right thalamic haemorrhage and hydrocephalous: a case report.

“…Moyamoya disease (MMD) is a rare bilateral steno occlusion of the internal carotid artery (ICA) (4), and it is an essential criterion on conventional angiography for the diagnosis (8). The disease mostly occurs in Japanese, Asians and nonCaucasians (3).…”